NKH Drugs. What and Why.
Updated: Mar 11, 2020
Sodium Benzoate or SB, strictly speaking, isn’t a medicine as such, it is actually a food preservative that is used in a few random disorders involving glycine. It was discovered that glycine sticks to Sodium Benzoate, so if you give large enough doses of it, when you pee out the sodium benzoate it takes the glycine from your blood with it. So naturally you only want to take this horrible preservative in massive doses if you have excess glycine floating around.
Here's some information on this pretty vile compound that our kids take multiple times a day just to keep them alive and functioning to some degree.
* Sodium benzoate is a preservative added to a variety of foods, beverages and condiments. You’ll even see it in shampoo and other beauty products. While it is generally recognized as safe in small doses, sodium benzoate may cause harmful if given in large doses. Which is of course awesome news for our children who take enormous amounts of this stuff.
* The most common source of sodium benzoate is food. The manufacturers use it as a preservative to prevent spoilage.
* Acidic products like sauerkraut, jellies and jams, hot sauce and soda are natural sources of sodium benzoate. Traces of sodium benzoate are present naturally in some foods and seasonings, including cranberries, cinnamon, prunes and apples.
* According to the December 2007 issue of "Environmental Health Perspectives" it has also been implicated as a potential trigger for hyperactivity in children with attention-deficit hyperactivity disorder.
* Sodium benzoate is used as a medication to treat hyperammonemia, a rare disorder that causes excess ammonia to accumulate in the blood. Its also used in Ketotic Hyperglycinemia (high glycine in the blood) and Non Ketotic Hyperglycinemia (high glycine in the blood and cerebral spinal fluid.
* 5mg/kg/day is recognised as safe to ingest in food. Dosing for NKH ranges from 200/mg/kg/day to 600mg/kg/day. Just let that sink in.
The second line of defense in NKH is Dextromethorphan or DXM. Whilst SB removes excess glycine from the blood, the real issue in NKH is excess glycine in the brain. To date no one has found how to remove it from the cerebral spinal fluid (CSF) but they have discovered that DXM can dull the receptors in the brain that the excess glycine is aggravating. If you think about excess glycine in your brain constantly causing misfiring and over stimulation of neurons by setting fires in your brain, then think of DXM as going around and putting out the fires. So it doesn’t address the cause but it can lessen the neurological effects. Of course there is far more to it but in simple terms its calming the crazy in the brain.
This drug is equally as lovely as SB. Here's some fun facts.
* Dextromethorphan isn’t a drug you can buy on its own but it is a compound found in multiple cough syrups. As such many NKH kids have to take huge amounts of cough syrup, several times a day. A bottle to last a day or two is not uncommon. In a few countries it is legal to have it made into a pure medicine.
* It is commonly taken recreationally to get high. DXM abuse is well documented. Lower amounts of it get you stoned, higher amounts of it and you “trip” and can achieve a dissociative state.
* It effects the brain much like ketamine and PCP (Angle Dust) does. Note the “meth” in its name.
* DXM is an Opioid, like Morphine.
* Prolonged abuse can cause impairment of memory and other mental functions. Large doses taken in conjunction with antidepressants and other drugs have caused death by respiratory and cardiac distress.
* Safe and recommended doses for theraputic use are 1 to 2mg/kg/day. In NKH doses can go as high as 15mg/kg/day.
Omeprazole is a Proton Pump Inhibitor (PPI). Other prescribed PPIS are lansoprazole (Prevacid), rabeprazole (Aciphex), pantoprazole (Protonix), and esomeprazole (Nexium). PPIs stop the stomach producing stomach acid and are widely used for acid reflux, stomach ulcers and other gastric conditions. The reason so many NKH children have to take a PPI is because SB burns the shit out of their insides. If you burp after SB, its like burping acid. In fact if you get any SB in a minor cut when preparing medicines, you soon know about it. It is virtually impossible to take SB as a medication if you don’t take it in conjunction with a PPI.
Omeprazole is dished out like candy by most doctors nowadays and if you, yourself arnt on it, I can almost guarantee that a friend or family member is. So it must be pretty harmless right?
* It massively upsets our natural gut bacteria. Studies have shown that people who regularly take omeprazole have different types of bacteria in their gut compared to those that don’t. People taking omeprazole have a higher risk of getting C. diff and E Coli.
* One study found a 70% increase in heart attacks and I much higher risk of kidney disease in those regularly taking a PPI.
* It it suggested that you only take a PPI for 14 days. NKH kids can take it their entire lives.
* Long term PPI use has been shown to cause malabsorption of key minerals in the body, particularly calcium and magnesium. Malabsorption of calcium causes significant bone problems and malabsorption of magnesium effects the heart, muscles and brain.
Virtually every NKH child will have needed a seizure medication at some point. I know of some children who are on five in total. However, two to three is probably the most common amount. Alice, fortunately, has not needed any since she was about four years old. We are in the tiny minority and we are forever grateful.
All seizure medications come with multiple side effects but as with all things in NKH, we don’t get to chose not to give these things. We simply have to give the least amount that we can get away with and way up the side effects over the possible outcomes if we don’t. Many of the more affected children have at some point been drugged to the point of unconsciousness with seizure medications, simply to stop the relentless and violent fits that NKH causes. This had to be done to Alice several times in her first few months of life.
Some seizure medications can actually prove fatal in NKH and I'll create a separate post of medications, foods and chemicals to avoid in NKH.